FEW PULMONARY MASSES CAN BE LIFE THREATENING: RAPIDLY PROGRESSING CASE OF GRANULOMATOSIS WITH POLYANGITIS

Abstract

SESSION TITLE: Diffuse Lung Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Granulomatosis with polyangiitis (GPA), formerly called Wegener Granulomatosis, is a necrotizing granulomatus inflammation-involving small to medium sized vessels. Early diagnosis and treatment is a key otherwise mortality and morbidity remains high from multi organ failure. Here we describe a rare case of 48 year old Hispanic male with rapidly progressing GPA who responded remarkably well to immunosuppressive therapy. CASE PRESENTATION: A 48-year-old Hispanic male was admitted for pneumonia and possible stroke symptoms. He had a 20-pound weight loss in addition to headaches, vision problems, and weakness. CT chest revealed multiple pulmonary masses. He was started on broad-spectrum antibiotics and then transferred to our facility for further care. Bronchoscopy showed predominant lymphocytes in bronchoalveolar lavage. Percutaneous biopsy of mass did not show changes of vasculitis. His respiratory status started to decline with hemoptysis. He also developed acute kidney injury that progressed to acute renal failure requiring hemodialysis. Proteinase 3 autoantibody (PR3) levels came back elevated. Based on his clinical presentation, diagnosis of GPA was suspected and he was started on IV steroids and Cyclophosphamide. He also got a single dose of Rituximab. Percutaneous renal biopsy was done which later on showed necrotizing cresentic pauci immune C-ANCA mediated glomerulonephritis. Eventually his renal function recovered and was taken off dialysis. He was discharged on oral steroids and immunosuppressive therapy to be managed by Rheumatology. DISCUSSION: Granulomatosis with polyangiitis (GPA) is usually more common in older adults especially in Caucasian population. Our case involved a Hispanic male. Studies have shown that Hispanic patients with these associated vasculitides have more severe disease and more extensive organ damage. We started immunosuppressive therapy based on clinical findings, anti-PR3 antibody without waiting for renal biopsy to save organs. CONCLUSIONS: While, the presenting symptoms of GPA can be vague and nonspecific, patient can rapidly progress to classical findings. Rapid diagnosis is a key since aggressive therapy is needed to help prevent multi-organ damage and subsequent failure. We recommend starting immunosuppressive therapy early without waiting for confirmatory biopsy if there is a threat to any of the organs and the clinical symptoms are highly suspicious for GPA. Reference #1: Flores-Suárez LF, Villa AR. Spectrum of Wegener granulomatosis in a Mexican population. Ann NY Acad Sci. 2007; 1107: 400-9. DISCLOSURES: No relevant relationships by Alexander Goldstein, source=Web Response No relevant relationships by Muhammad Malik, source=Web Response No relevant relationships by Kim Nguyen, source=Web Response No relevant relationships by Manish Patel, source=Web Response No relevant relationships by Grace Wang, source=Web Response